There are many types and subtypes of autoimmune hemolytic anemia (AIHA). When it occurs itself with no underlying disease, it’s called primary or idiopathic AIHA. When it’s caused by other diseases, it’s called secondary AIHA. Among diseases that can cause AIHA is systemic lupus erythematosus (SLE or lupus). Lupus is a chronic disease that causes inflammation and pain.

AIHA occurs in approximately 5 percent to 10 percent of people with lupus. In some people, AIHA may be the first sign of lupus, and it may appear several years before a person is diagnosed with lupus.

How Lupus Can Cause AIHA

Hematological abnormalities (problems with the blood and blood-forming organs) are common in people living with lupus. One of these abnormalities is AIHA. AIHA may be caused by lupus or lupus treatments.

Lupus Itself May Cause AIHA

The body produces antibodies (also called immunoglobulins). These are proteins the immune system uses to identify and destroy toxins and foreign substances. These foreign substances, such as viruses or bacteria, contain molecules known as antigens. Antibodies can bind to antigens to destroy them or signal the immune system to attack.

Lupus is an autoimmune disease, meaning that the body mistakes healthy cells for potentially harmful antigens. As a result, the immune system may end up producing autoantibodies, which are antibodies that attack substances in a person’s own body. These autoantibodies can destroy healthy red blood cells, causing autoimmune hemolysis (destruction of red blood cells).

Lupus Treatments May Cause AIHA

AIHA may be caused by medications. This is known as drug-induced immune hemolytic anemia (DIIHA). Although DIIHA is rare and manageable with proper treatment, it’s important to be aware of the potential risks.

A drug can cause AIHA by causing the immune system to accidentally identify red blood cells as foreign substances. The body then makes antibodies to attack red blood cells, causing them to break down.

Certain nonsteroidal anti-inflammatory drugs (NSAIDs) have been shown to cause AIHA. NSAIDs are used to relieve lupus symptoms by reducing inflammation that causes stiffness and pain in the muscles and joints. Examples of NSAIDs include ibuprofen (sold as Advil and Motrin) and diclofenac (including Flector, Cambia, and Zipsor).

One study found that, among 73 people who developed DIIHA between 1996 and 2015, 23 cases were linked to diclofenac and one was linked to ibuprofen. Scientists believe AIHA can occur when the body is exposed to an NSAID or its metabolites (byproducts of the body breaking down a drug), which leads to the development of autoantibodies and antibodies.

How To Treat AIHA if You Also Have Lupus

There is not a lot of research regarding the best way to treat AIHA and lupus.

Clinical trials — research studies involving people to gather medical data — that have examined AIHA treatments have primarily studied only idiopathic AIHA. As a result, there’s not much strong scientific evidence about how to treat people with lupus and AIHA — especially for those with AIHA that’s refractory (unresponsive to treatment).

There are no official guidelines to treat people with both conditions, but doctors may prescribe the following treatments.

First-Line Therapy

The first-line or preferred therapy for AIHA is usually a glucocorticoid (also known as a corticosteroid). These medications help decrease swelling and pain and are commonly used to treat lupus and AIHA. Examples include prednisone or methylprednisolone.

Second-Line Therapy

If a person with AIHA doesn’t respond to the first-line treatment, they have refractory AIHA. In this case, the doctor will move on to second-line therapy.

Rituximab (Rituxan) is commonly used in treating refractory AIHA. Rituximab is a biologic monoclonal antibody that’s approved by the U.S. Food and Drug Administration (FDA) to treat several conditions, including non-Hodgkin lymphoma, chronic lymphocytic leukemia, and rheumatoid arthritis. However, doctors sometimes prescribe rituximab off-label, meaning for conditions beyond those indicated by the FDA. AIHA is one such condition.

Third-Line Therapy

If rituximab isn’t effective either, the doctor may suggest a splenectomy. This surgical procedure involves removing the spleen, the organ that filters blood and helps the body fight germs and infections.

Third-line medications include immunosuppressive therapy, which aims to decrease activity in the immune system. These medications include:

• Mycophenolate mofetil (CellCept)
• Azathioprine (including Imuran and Azasan)
• Cyclophosphamide (including Cytoxan and Neosar)

These medications can also be used for treating severe lupus symptoms.

Potential Complications if You Have Lupus and AIHA

Complications are medical problems that occur during the course of a disease. AIHA destroys red blood cells, a process known as hemolysis. Hemolysis, as well as the resulting lack of red blood cells (known as anemia), can cause complications.

AIHA that is caused by lupus will also be associated with complications of lupus.

Complications From Hemolysis and Anemia

The destruction of red blood cells can lead to symptoms of anemia, including fatigue, shortness of breath, and other complications.

Rarely, people with AIHA can develop skin complications. Usually seen in those with cold AIHA, the lack of blood flow to the extremities can lead to experiencing numbness and pain and feeling cold. Hemoglobin, a protein found in red blood cells, is responsible for carrying oxygen. Without enough hemoglobin and oxygen, the skin can become pale or even blue. In severe cases, the skin cells may die — known as cutaneous necrosis. These skin complications may be avoided by keeping extremities, especially the hands and feet, warm.

Another complication of AIHA is folate deficiency. With constant hemolysis, the body tries to compensate for the lost red blood cells by producing more. The process of generating new red blood cells requires vitamin B9 — also known as folate. The increased demand for folate can result in a deficiency. As a result, doctors sometimes prescribe or recommend folic acid supplements for people with AIHA. Always speak with your health care provider before starting a new supplement.

AIHA has been linked to an increased risk of venous thromboembolism, which is when blood clots form in the veins. Approximately 10 percent to 27 percent of people with AIHA have experienced venous thromboembolism.

Constant and uncontrolled hemolysis in people with AIAH may lead to acute (short-term) kidney injury. Destroyed red blood cells are cleared through the kidneys. In AIHA, these cell parts may start accumulating and cause stress and damage to the kidneys.

Complications From Underlying Lupus

Osteoporosis is a complication of lupus, especially in people who’ve been treated with corticosteroids, as osteoporosis is a side effect of these medications. Osteoporosis is a condition in which the bones become weak, which makes them more likely to break.

Lupus can affect the kidneys as well. Serious kidney damage can occur when the immune system attacks the kidneys. This is known as lupus nephritis.

People with lupus are at a higher risk for developing atherosclerosis and atherosclerotic cardiovascular disease. Atherosclerosis is the narrowing of arteries due to the buildup of plaque (made up of fats, cholesterol, and other substances). It can lead to heart attacks and strokes.

Lupus can increase inflammation in the lining of the chest cavity, which can lead to painful breathing. Pneumonia or bleeding into the lungs can occur.

People with lupus are more likely to develop infections. Lupus itself, as well as its treatments, can weaken the immune system, making it harder for the body to fight off infections.

Complications From Lupus and AIHA

One study of 870 people with lupus found that those who also had AIHA were more likely to have leukopenia (decreased white blood cell count) and thrombocytopenia (decreased platelet count).

A 2008 study in the journal Lupus looked at 76 females with both lupus and AIHA. Compared to people with lupus but not AIHA, this group was more likely to experience the following:

• Pericarditis — Inflammation of the pericardium, which is a saclike membrane surrounding the heart
• Pleuritis (also called pleurisy) — Inflammation of the tissues that separate the lungs from the chest wall
• Lupus nephritis — Kidney disease caused by lupus
• Seizures — A sudden burst of uncontrolled electrical activity in the brain

Communicate With Your Health Care Provider

Talk to your doctor if you have any questions about AIHA and lupus. If you are living with both conditions, your doctor can go over the different treatment options with you and develop a plan to help relieve your symptoms. Make sure to attend follow-up appointments.

Talk With Others Who Understand

On myAIHAteam, the social network for people with autoimmune hemolytic anemia and their loved ones, members come together to ask questions, give advice, and share their stories with others who understand life with AIHA.

Are you living with AIHA and lupus? Share your experience in the comments below, or start a conversation by posting on your Activities page.